PubMed · 7278340
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V H Kaunitz. 1981. Flail chest.. https://pubmed.ncbi.nlm.nih.gov/7278340/
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We describe two boys with the cerebro-costo-mandibular syndrome (CCMS). Both patients presented with Pierre Robin anomaly and respiratory insufficiency and died 12 hours and 10 months after birth. The first boy had muscular hypotonia, severe micrognathia, glossoptosis, short palate, preauricular tag, paraumbilical fibroma, and a small and narrow thorax. His chest roentgenographs showed marked hypoplasia of the first to tenth rib, multiple posterior rib-gaps in the only four ossified ribs. Tracheomalacia and stenosis of the left ureter was observed during autopsy. No structural cerebral anomalies were observed. Respiratory distress necessitated a tracheostomy in the second boy. He had severe micrognathia with glossoptosis and a cleft soft palate were noted. His chest roentgenograph showed a bell-shaped, small thorax with multiple dorsal rib-gap defects. CCMS is a rare disorder often associated with Pierre Robin anomaly. Chest roentgenographs show the typical posterior rib-gap defects, which are quite variable. CCMS usually occurs as an isolated event in a family. Of 41 reported families four reports describe horizontal and two describe vertical transmission of CCMS. This might imply genetic heterogeneity with autosomal recessive and autosomal dominant inheritance. Inter- and intrafamilial expression is variable. Careful family studies are necessary before genetic counseling is given.
Thoracic trauma represents 12.8% of all trauma patients treated in author's evaluated the contribution of new diagnostic methods, in particular CT and sonography, to determinate the indication of conservative therapy or early surgical treatment. From 1992 in the framework of the grant project the early surgical treatment of instable thorax was performed in 29 patients with polytrauma or severe associated thoracoabdominal trauma. Authors determined indications for thorax stabilisation after serious consideration, and stated contemporary contraindications of this treatment. They evaluated the therapeutic contribution of these methods and prepared in cooperation with the company Medin Nové Mĕsto the set of osteosynthetic material with accessories for treatment of instable thorax.
The authors make a wide-ranging review of the literature, examine the epidemiological aspect of fail chest and underline the importance of its correct anatomopathological classification for a more qualified physiopathologist, diagnostic and therapeutic approach. They make a historical excursion on volet's treatment and underline the complementarity of surgical and resuscitation therapies.